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Department of Psychiatry, University of Rochester Medical Center, Rochester, NY (Ms. Bamford and Dr. Caine)
Department of Neurology, University of Rochester Medical Center, Rochester, NY (Drs. Caine and Shoulson)
Department of Radiology, University of Rochester Medical Center, Rochester, NY (Drs. Kido and Plassche)
Department of Medicine, University of Rochester Medical Center, Rochester, NY (Dr. Shoulson)
To examine the premise that cognitive impairment in Huntington's disease (HD) is related to striatal degeneration, we determined those cognitive deficits most closely associated with linear CT indices of brain atrophy in HD. We systematically evalated 60 drug-free HD patients who were judged to be in stages I (n = 34) or II (n = 26) of illness. All subjects underwent comprehensive neuropsychological assessment covering a broad spectrum of cognitive operations and standardized head CT imaging for determination of frontal horn (FH), intercaudate (CC), and outer-table (OT) distances. We grouped the cognitive test results, based on a principal-component factor analysis, to form factors 1 (complex psychomotor), 2 (verbal memory), 3 (visuospatial), and 4 (general knowledge). Factors 1 and 3 sharply discriminated between subjects in stages I and II of illness. Factors 1, 2, and 3 correlated strongly with CC/OT, an index of caudate atrophy, whereas only factor 2 correlated with FH/OT, an index of frontal atrophy. These data demonstrate that cognitive impairment is a clear-cut characteristic of early HD that is linked closely to the extent of caudate atrophy as measured by CT.
Address correspondence and reprint requests to Dr. Caine, University of Rochester Medical Center, 300 Crittenden Boulevard, Rochester, NY 14642.
Supported in part by grants NS 17978, MH 00473, and MH 40381; and RR 00044, to the University of Rochester Clinical Research Center.
Received August 4, 1988. Accepted for publication in final form January 9, 1989.
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